Risk factors and impact of allergic bronchopulmonary aspergillosis in <i>Pseudomonas aeruginosa‐</i>negative <scp>CF</scp> patients

Baets, Frans De; Keyzer, L. De; Daele, S. Van; Schelstraete, Petra; Biervliet, S. Van; Braeckel, Eva Van; Thomas, M.; Wanyama, S.

doi:10.1111/pai.12953

Cited by 8 publications

(7 citation statements)

References 12 publications

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“…Aspergillus fumigatus is a common fungal pathogen isolated from CF airways [ 2 ]. Its presence can lead to the development of allergic bronchopulmonary aspergillosis (ABPA), which has been reported to have a negative impact on lung function in CF [ 117 , 118 ]. Studies utilising registry databases indicate a beneficial role of IVA on the colonisation of Aspergillus in the CF lung [ 119 , 120 ], reducing the opportunity for the establishment of airway infection ( Table 1 ).…”

Section: Impact Of Cftr Modulators On Airway Infectionmentioning

confidence: 99%

The Effect of CFTR Modulators on Airway Infection in Cystic Fibrosis

Harvey

Weldon

Elborn

et al. 2022

IJMS

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The advent of Cystic fibrosis transmembrane receptor (CFTR) modulators in 2012 was a critical event in the history of cystic fibrosis (CF) treatment. Unlike traditional therapies that target downstream effects of CFTR dysfunction, CFTR modulators aim to correct the underlying defect at the protein level. These genotype-specific therapies are now available for an increasing number of CF patients, transforming the way we view the condition from a life-limiting disease to one that can be effectively managed. Several studies have demonstrated the vast improvement CFTR modulators have on normalization of sweat chloride, CFTR function, clinical endpoints, and frequency of pulmonary exacerbation. However, their impact on other aspects of the disease, such as pathogenic burden and airway infection, remain under explored. Frequent airway infections as a result of increased susceptibility and impaired innate immune response are a serious problem within CF, often leading to accelerated decline in lung function and disease progression. Current evidence suggests that CFTR modulators are unable to eradicate pathogenic organisms in those with already established lung disease. However, this may not be the case for those with relatively low levels of disease progression and conserved microbial diversity, such as young patients. Furthermore, it remains unknown whether the restorative effects exerted by CFTR modulators extend to immune cells, such as phagocytes, which have the potential to modulate the response of people with CF (pwCF) to infection. Throughout this review, we look at the potential impact of CFTR modulators on airway infection in CF and their ability to shape impaired pulmonary defences to pathogens.

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Section: Impact Of Cftr Modulators On Airway Infectionmentioning

confidence: 99%

The Effect of CFTR Modulators on Airway Infection in Cystic Fibrosis

Harvey

Weldon

Elborn

et al. 2022

IJMS

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“…ABPA is a serious complication in PwCF and the result of a complex immunologic response against A. fumigatus. ABPA has a prevalence of 4-9% in children and around 10% in adult PwCF [11,[82][83][84][85][86][87][88]. ABPA individuals sensitised to A. fumigatus can develop an IgE-mediated allergic inflammatory reaction (type I immediate hypersensitivity) which leads to an acute inflammatory response resulting in mucus impaction and bronchial obstruction after inhaling A. fumigatus spores [89][90][91].…”

Section: Allergic Pulmonary Aspergillosis Infection (Abpa)mentioning

confidence: 99%

Aspergillus-related lung disease in people with cystic fibrosis: can imaging help us to diagnose disease?

Elders

Warris

et al. 2021

Eur Respir Rev

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In people with cystic fibrosis (PwCF), viscous sputum and dysfunction of the mucociliary escalator leads to early and chronic infections. The prevalence of Aspergillus fumigatus in sputum is high in PwCF and the contribution of A. fumigatus to the progression of structural lung disease has been reported. However, overall, relatively little is known about the contribution of A. fumigatus to CF lung disease. More knowledge is needed to aid clinical decisions on whether to start antifungal treatment. In this review, we give an overview of A. fumigatus colonisation and infection in PwCF and the different types of pulmonary disease caused by it. Furthermore, we discuss the current evidence for structural lung damage associated with A. fumigatus in PwCF on chest computed tomography and magnetic resonance imaging. We conclude that radiological outcomes to identify disease caused by A. fumigatus can be important for clinical studies and management.

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“…aeruginosa- negative patients and found that patients with ABPA had significantly lower FEV1 values. They suggested that this decline in lung function preceded ABPA diagnosis by approximately 2 years [ 39 ]. When other factors were analysed, a low BMI Z-score was found to have the greatest impact on the progression of lung disease followed by high-risk genotype, female sex, CF-related diabetes mellitus, chronic P .…”

Section: Abpa and Lung Functionmentioning

confidence: 99%

Allergic Bronchopulmonary Aspergillosis in Children with Cystic Fibrosis: An Update on the Newest Diagnostic Tools and Therapeutic Approaches

et al. 2020

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Cystic fibrosis (CF), the most common autosomal-recessive genetic disease in the Caucasian population, is characterized by frequent respiratory infections and progressive lung disease. Fungal species are commonly found in patients with CF, and among them, Aspergillus fumigatus is the most frequently isolated. While bacteria, particularly Pseudomonas aeruginosa, have a well-established negative effect on CF lung disease, the impact of fungal infections remains unclear. In patients with CF, inhalation of Aspergillus conidia can cause allergic bronchopulmonary aspergillosis (ABPA), a Th2-mediated lung disease that can contribute to disease progression. Clinical features, diagnostic criteria and treatment of ABPA are still a matter of debate. Given the consequences of a late ABPA diagnosis or the risk of ABPA overdiagnosis, it is imperative that the diagnostic criteria guidelines are reviewed and standardized. Along with traditional criteria, radiological features are emerging as tools for further classification as well as novel immunological tests. Corticosteroids, itraconazole and voriconazole continue to be the bedrock of ABPA therapy, but other molecules, such as posaconazole, vitamin D, recombinant INF-γ and Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) modulators, have been showing positive results. However, few studies have been conducted recruiting CF patients, and more research is needed to improve the prevention and the classification of clinical manifestations as well as to personalize treatment. Early recognition and early treatment of fungal infections may be fundamental to prevent progression of CF disease. The aim of this narrative review is to give an update on ABPA in children with CF.

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Risk factors and impact of allergic bronchopulmonary aspergillosis in Pseudomonas aeruginosa‐negative CF patients

Cited by 8 publications

References 12 publications

The Effect of CFTR Modulators on Airway Infection in Cystic Fibrosis

The Effect of CFTR Modulators on Airway Infection in Cystic Fibrosis

Aspergillus-related lung disease in people with cystic fibrosis: can imaging help us to diagnose disease?

Allergic Bronchopulmonary Aspergillosis in Children with Cystic Fibrosis: An Update on the Newest Diagnostic Tools and Therapeutic Approaches

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